Squamous Cell Carcinoma of the Kidney: A Large Case Series.

OBJECTIVES: We present our experience with and data about a very rare neoplasm of the kidney, squamous cell carcinoma (SCC). METHODS: A total of 14 patients with a diagnosis of SCC were identified on the basis of a retrospective analysis of medical records of patients who underwent surgery for renal cancers between 2015 and 2021 at the Sindh Institute of Urology and Transplantation. IBM SPSS v25 was used to record and analyze data. RESULTS: Most patients found to have SCC of the kidney were male (71.4%). The mean (SD) patient age was 56 (13.7) years. Flank pain was the most common presenting symptom (n = 11; 78.6%) followed by fever (n = 6; 42.9%). Only 4 (28.5%) of the 14 patients had a preoperatively established diagnosis of SCC; the remaining 10 (71.4%) had an incidental finding of SCC on their histopathology specimen. The mean (SD) overall survival was 5 (4.5) months. CONCLUSIONS: SCC of the kidney is a rare upper urinary tract neoplasm reported in the literature. The gradual onset of vague symptoms, lack of pathognomonic signs, and inconclusive radiological features make the disease unsuspected in most cases, therefore delaying diagnosis and treatment. It usually presents at an advanced stage, and the prognosis is often poor. A high index of suspicion is warranted in patients with chronic kidney stone disease.

Chondrosarcoma of right big toe with metastases to left orbital and left infra temporal region.

To report a case of chondrosarcoma of right big toe with left orbital and left infra temporal metastases. Chondrosarcoma is the second most frequent primary malignant tumour of the bone. A 56 year old man had history of trauma on his right big toe, which was amputated and the biopsy in 2011 at Sindh Institute of Urology and Transplantation (SIUT) revealed chondrosarcoma with clear margins. Eventually the patient was presented with swelling of the left eye, pain and gradual loss of vision of that eye. Later a CT scan of his chest, brain and orbit showed pulmonary and pleural based nodule, with mediastinal and hilar lymphadenopathy representing metastatic deposit in left orbit, extending to left infra temporal region. A treatment of palliative chemotherapy was started with doxorubicin and ifosfamide, after which he was referred for radiotherapy. At that time he had loss of vision, pain and exopthalamus, and palliative radiotherapy was delivered to the left orbit with the prescribed dose of30 Gy/300cGy×10 fraction. Thereafter his case will be followed up at the oncology OPD after a 03 month interval.

Early post-operative outcomes of robot-assisted pyeloplasty in patients with unilateral ureteropelvic junction obstruction.

INTRODUCTION: Ureteropelvic junction obstruction (UPJO) is a commonly encountered abnormality and it can lead to serious consequences such as renal dysplasia eventually resulting in loss of kidney. Hence, early diagnosis and timely management remains the cornerstone of the treatment. The most anticipated technique amongst modern day urologist is the robot-assisted laparoscopic pyeloplasty (RALP). The study aims to determine early post-operative outcomes of robot-assisted laparoscopic transperitoneal pyeloplasty procedure in patients presenting with unilateral ureteropelvic junction obstruction to establish the local perspective. METHODOLOGY: This is a descriptive study involving patients with ureteropelvic junction obstruction in a tertiary care facility in Karachi; Sindh Institute of Urology and Transplant (SIUT). A total of 46 participants were recruited. Robot-assisted laparoscopic transperitoneal dismembered Hynes-Anderson pyeloplasty was performed by a single surgeon with over 3 years of experience in the presence of the researcher. Early postoperative outcome total operative time, length of hospital stay, console time and blood loss were noted by the researcher as per operational definition. Data were analyzed on SPSS Version 22. RESULTS: Mean age in our study was 46.51 years with the standard deviation of ± 10.87. Whereas, mean length of hospital stay, total operative time, total blood loss, console time, pre-hemoglobin, posthemoglobin, height, weight and BMI in our study was 1.19 ± 0.40 days, 64.58 ± 17.59 min, 9.56 ± 6.13 ml, 30.17 ± 4.99 min, 12.66 ± 1.47 ml, 11.79 ± 1.93 ml, 165.62 ± 8.23 cm, 68.34 ± 8.23 kg and 24.85 ± 3.34 kg/m2, respectively. CONCLUSION: Recent advancements in technology have yielded the latest RALP technique which has been proven significantly better than existing approaches and similar results are reported by this study demonstrating improvement in peri-operative and post-operative outcomes ultimately ameliorating the quality of life of patients with UPJO.

Da Vinci Robotic Surgical System: Early Experience from Pakistan.

OBJECTIVE: To determine the outcomes of robotic surgeries using the Da Vinci robotic surgical system (DVSS) at Pakistan's largest urological tertiary healthcare system. STUDY DESIGN: Descriptive study. Place and Duration of the Study: Department of Minimally Invasive and Robotic Surgery, Division of Urology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan, from March 2017 to December 2021. METHODOLOGY: Hospital records of patients who underwent robotic urological surgeries were assessed for their outcomes in terms of blood loss, mortality, system malfunction, and conversion to open surgery. Descriptive statistics were determined. RESULTS: A total of 550 procedures were performed. The mean age recorded was 39.58±16.83 years. The mean blood loss recorded was 255.611±353.57 ml; there were 3 cases of malfunction. Forty cases were converted to open surgery, and the mortality rate was 1.1%. CONCLUSION: Minimally invasive surgical techniques have high precision, fewer complications, and lower morbidity rates. Using DVSS for surgical interventions is both effective and safe. KEY WORDS: Minimally invasive surgery, Da Vinci robotic surgical system, Robotic surgery.

Comparison of resistive index and shear-wave elastography in the evaluation of chronic kidney allograft dysfunction.

BACKGROUND: Detection of early chronic changes in the kidney allograft is important for timely intervention and long-term survival. Conventional and novel ultrasound-based investigations are being increasingly used for this purpose with variable results. AIM: To compare the diagnostic performance of resistive index (RI) and shear wave elastography (SWE) in the diagnosis of chronic fibrosing changes of kidney allograft with histopathological results. METHODS: This is a cross-sectional and comparative study. A total of 154 kidney transplant recipients were included in this study, which was conducted at the Departments of Transplantation and Radiology, Sindh Institute of Urology and Transplan tation, Karachi, Pakistan, from August 2022 to February 2023. All consecutive patients with increased serum creatinine levels and reduced glomerular filtration rate (GFR) after three months of transplantation were enrolled in this study. SWE and RI were performed and the findings of these were evaluated against the kidney allograft biopsy results to determine their diagnostic utility. RESULTS: The mean age of all patients was 35.32 ± 11.08 years. Among these, 126 (81.8%) were males and 28 (18.2%) were females. The mean serum creatinine in all patients was 2.86 ± 1.68 mg/dL and the mean estimated GFR was 35.38 ± 17.27 mL/min/1.73 m2. Kidney allograft biopsy results showed chronic changes in 55 (37.66%) biopsies. The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of SWE for the detection of chronic allograft damage were 93.10%, 96.87%%, 94.73%, and 95.87%, respectively, and the diagnostic accuracy was 95.45%. For RI, the sensitivity, specificity, PPV, and NPV were 76.92%, 83.33%, 70.17%, and 87.62%, respectively, and the diagnostic accuracy was 81.16%. CONCLUSION: The results from this study show that SWE is more sensitive and specific as compared to RI in the evaluation of chronic allograft damage. It can be of great help during the routine follow-up of kidney transplant recipients for screening and early detection of chronic changes and selecting patients for allograft biopsy.

Transition from da Vinci to Versius robotic surgical system: initial experience and outcomes of over 100 consecutive procedures.

We sought to describe the development of the robotic urology program at Sindh Institute of Urology and Transplantation (SIUT) and the feasibility of transitioning from the da Vinci to Versius robotic systems. The SIUT robotics program began in 2017 utilizing the da Vinci Si robotic system, transitioning to the Versius system in 2021. Retrospective review of our quality assurance database was performed. All procedures performed utilizing the two systems were identified, analyzed, and compared. Data were described with descriptive statistics. Matched procedures (by type of procedure) performed by the same surgeons utilizing the da Vinci and Versius were compared. All tests were double-sided with statistical significance set at p < 0.05.106 cases were performed by the Versius robotic surgical system in 2021. Median age was 42 years (IQR 26-56), and 69 (65%) were males. Procedures included both benign (83%) and malignant disorders (17%), several upper tract (75%), and pelvic/lower tract (25%). No major intraoperative complications were observed. Conversion to open occurred in six procedures. Malfunction of the robotic arms occurred in two procedures: the erroneous bedside units (BSU) were replaced. Eight patients developed postoperative high-grade complications. Matched analysis of various procedures (pyeloplasty, stone surgery, radical, partial, and simple nephrectomy) showed no significant difference in perioperative outcomes. To our knowledge, this is the first and largest series of urologic procedures performed by the Versius robotic surgical system.

Outcomes of Nephron-sparing Surgery in T1 vs T2 Renal Tumours.

OBJECTIVE:  To compare the drop in eGFR after nephron-sparing surgery in T1 and T2 renal tumours. STUDY DESIGN:  Descriptive study. PLACE AND DURATION OF STUDY:  Department of Uro-oncology, Sindh Institute of Urology, and Transplantation Karachi, from March 2020 to March 2021. METHODOLOGY:  Retrospective data were collected for all patients who underwent nephron-sparing surgery between 2014 to 2019. Eighty-seven patients were divided into two groups based on the T stage of renal tumours (T1 ≤7 cm and T2 >7 cm). The outcomes of the two groups were compared such as eGFR, blood transfusion, hospital stay and complications. RESULTS:  There was a higher drop in eGFR in T2 tumours when compared to T1 tumours at 1 year of follow-up. There were more perioperative complications, higher blood transfusions and longer hospital stays for T2 tumours. CONCLUSION:  Nephron sparing surgery for T2 renal tumours carries lower eGFR preservation, higher blood transfusions and complications when compared to T1 tumours. The indication for such extensive surgery should be individualized to specific contexts only. KEY WORDS: Adenocarcinoma kidney, Nephrectomy, Glomerular filtration rate, Length of hospital stay, Blood transfusion.

Excision of Postchemotherapy Residual Retroperitoneal Mass in Testicular Cancer.

In this study, patients, who underwent excision of retroperitoneal mass following chemotherapy for testicular cancers from 2006 to 2016, were studied and followed till 2021. The clinical and oncological outcomes were measured. Among 338 patients, who were treated for TC during the entire study period, 38 (11.2%) underwent excision of the residual retroperitoneal mass. The mean age of these patients was 26.9±6.3 years. The majority were stage 3 testicular cancer. Complete resection with negative microscopic margins was achieved in the majority of cases i.e. 31 (81.6%). Damage to the collateral structures was seen in 12 (31.6%) cases. On histopathological examination, teratoma was seen in 18 (47.4%) of the cases followed by necrosis in 10 (26.3%). Complications were recorded in 12 (31.5%) cases, most were low grades. At five years of follow-up, 19 (50%) were disease-free, 25 (65.8%) were alive, and 9 (23.6%) were lost to follow-up. Five-year survival rates are lower than standard template-based retroperitoneal dissection, however, complications rates are comparable. Such complex surgeries should only be performed in high-volume centres. Key Words: Testicular neoplasms, Lymph node excision, Seminoma, Germ cell and embryonal.

Outcome of trimodality protocol for invasive bladder cancer patients at Karachi, Pakistan.

OBJECTIVES: To evaluate the outcomes of patients with muscle invasive bladder cancer managed by trimodality protocol with assessment of factors that may predict treatment response, risk of recurrences and survival of such patients in our population. METHODS: A prospective, single arm study was conducted between July 2006 and December 2009 at the Sindh Institute of Urology and Transplantion, Karachi. One hundred and sixteen patients with muscle invasive bladder cancer T2-T3N0M0 were treated with concurrent chemoradiation (total dose 6500 cGy) after maximal transurethral resection. Complete response was defined as no tumour seen on check cystoscopy and biopsy. The disease control and overall survival were determined by Kaplan and Meier method and statistical inferences with the log-rank test. Cox regression analysis was used to find different prognostic factors. RESULTS: At the median follow up of 36 months (14-43), out of total 116, 62(51.6%) surviving patients the bladder was functioning well, while 18(15%) had local recurrence; in 9 patients superficial tumour recurred and required further transurethral resection and intravesical drug therapy and nine patients who had muscle invasive recurrence; underwent radical cystectomies. Concurrent chemoradiation was well tolerated. The overall survival at 3 years was 54%. Initial complete response, primary tumour stage and absence of hydronephrosis were the most important prognostic factors for survival (all p=<0.0001). CONCLUSION: Trimodality treatment was found to be an effective therapy in patients with invasive bladder cancer and complete TURBT, tumour stage and no hydronephrosis at time of presentation were found important prognostic factors for treatment response, disease free and over all survival rates.

Adrenal myelolipoma: a report of three cases and review of literature.

Adrenal myelolipoma is a rare and benign tumour composed of mature adipose tissue and haematopoietic elements that resemble bone marrow. It is mostly discovered incidentally on imaging of abdomen done for non adrenal related reasons or at autopsy. Usually asymptomatic, but has been reported to present with symptoms such as flank pain resulting from tumour bulk, necrosis or spontaneous retroperitoneal haemorrhage. Symptomatic tumours, growing tumours or tumours larger than 10 cm should be excised surgically. We report cases of 3 male patients presenting with flank pain and upper pole renal masses. All three were treated surgically with adrenalectomy. Myelolipoma was confirmed in all three on histology.

Laparoscopic Donor Nephrectomy: Early Experience at a Single Center in Pakistan.

OBJECTIVES: Laparoscopic donor nephrectomy has become the criterion standard for kidney retrieval from living donors. There is no information on the experience and outcomes of laparoscopic donor nephrectomy in Pakistan. The objective of the study was to identify benefits and harms of using laparoscopic compared with open nephrectomy techniques for renal allograft retrieval. MATERIALS AND METHODS: In this a retrospective study, patient files from May 2014 to September 2015 were analyzed. Patients were divided into 2 groups: those with open donor nephrectomy and those with laparoscopic donor nephrectomy. Donor case files and operative notes were analyzed for age, sex, laterality, body mass index, warm ischemia time, perioperative and postoperative complications, surgery time, and length of hospital stay. Finally, serum creatinine patterns of both donors and recipients were analyzed. Data were analyzed using SPSS version 10 (SPSS: An IBM Company, IBM Corporation, Armonk, NY, USA). RESULTS: Of 388 total donors, 190 (49%) had open donor nephrectomy and 198 (51%) had laparoscopic donor nephrectomy. For both groups, most donors were older than 25 years with male preponderance. Left-to-right kidney donation ratio was markedly higher in the laparoscopic group than in the open donor nephrectomy group, with 6 cases of double renal artery also included in this study. There were no significant differences in surgery times between the 2 groups, whereas the laparoscopic donor nephrectomy group had shorter hospital stay. Analgesic requirements were markedly shorter in the laparoscopic donor nephrectomy group. The 1-year graft function was not significantly different between the 2 groups. CONCLUSIONS: The results for laparoscopic donor nephrectomy were comparable to those for open donor nephrectomy, and its acceptability was high. Laparoscopic donor nephrectomy should be the preferred approach for procuring the kidney graft.

Kidney Swap in Pakistan: Experience at the Sindh Institute of Urology and Transplantation.

Kidney paired exchange is an established method of overcoming incompatibility in donor-recipient pairs and expanding the living-donor pool. It is infrequently performed in developing countries. We report the first kidney paired exchange in Pakistan, successfully performed at our center. One donor-recipient pair consisted of a 38-year-old female recipient (blood type, B positive) and her 40-year-old husband (A positive) as the potential donor. The second pair consisted of a 30-year-old male recipient (A positive) and his 30-year-old wife (B positive) as the potential donor. The donors were exchanged with the recipients, and both pairs were antigen matched for human leukocyte antigen A and human leukocyte antigen DR. Luminex antibody screening was negative, as were the crossmatches for T and B cells and for IgG and IgM. The transplant procedures and recoveries proceeded uneventfully. The recipients are maintaining serum creatinine levels around 0.78 mg/dL and 0.90 mg/dL, 1 year after transplant. Kidney paired exchange is a relatively low-cost option for overcoming the barrier of incompatibility in a resource-constrained setting.

Analysis of the glutathione S-transferase genes polymorphisms in the risk and prognosis of renal cell carcinomas. Case-control and meta-analysis.

BACKGROUND: The Glutathione S-transferases (GSTs) genes deletion polymorphisms have been associated with the progression of several cancers. The association studies between the 2 GSTs (GSTM1 and GSTT1) null polymorphisms with the susceptibility to renal cell carcinoma (RCC) have been inconclusive. Therefore, with the inclusion of our own data, we performed a comprehensive meta-analysis to assess the association between these 2 polymorphisms and the risk of RCC. METHODS: A systematic literature search was carried out for studies published in the PubMed, EMBASE, Cochrane library, and Google Scholar from 1997 to December 2014. Results were stated as pooled odds ratios (ORs) for nonparametric data after heterogeneity analysis with 95% CI using fixed effect or random effect model. RESULTS: We systematically selected 13 relevant studies after thorough searches from the databases. Data showed no association between the GSTM1 and the GSTT1 null genotypes and the risk of RCC (OR = 1.01; CI: 0.92-1.11; P = 0.89 for GSTM1 and OR = 1.14; CI: 0.91-1.42; P = 0.25 for GSTT1). No association was found when the data were stratified according to the geographical/ethnic basis, source of control, and the risk factor evaluation. Subgroup analysis of occupational exposure to pesticides showed an inverse association of the active genotypes of both GSTM1 and GSTT1 polymorphisms with the exposed group of RCC (P<0.00001 and P<0.00001, respectively). The combined null genotype of the GSTM1/GSTT1 significantly increased the susceptibility to RCC by 1.4-fold (P = 0.001). This association remained significant for the Asian populations in subgroup analysis (OR = 1.8; CI: 1.30-2.49; P = 0.0004). CONCLUSION: In conclusion, this meta-analysis suggests that the 2 GSTs deletion polymorphisms independently have no association with the risk of RCC. However, combination of both deletions increases the risk of developing the RCC.

Unique molecular alteration patterns in von Hippel-Lindau (VHL) gene in a cohort of sporadic renal cell carcinoma patients from Pakistan.

BACKGROUND: Renal cell carcinoma (RCC) is the most frequent form of kidney cancer in adults. Somatic mutations that inactivate the von Hippel-Lindau (VHL) gene are the most common cause of RCC. The frequencies of molecular changes in the VHL gene in RCCs vary among different populations. So far, a single chromosomal-based study has been reported from a South Asian population. This report presents, for the first time, the somatic changes and promoter hypermethylation in VHL in a cohort of 300 RCC patients from Pakistan. METHODS: To identify mutations in the VHL gene, direct DNA sequencing was carried out. Epigenetic silencing was investigated by using methylation-specific polymerase chain reaction. RESULTS: Our data showed molecular alterations in the VHL gene in 163 (54%) renal cell carcinoma patients. Somatic mutations were found in 87 (29%) patients and 35 novel mutations were identified. VHL promoter hyper-methylation analysis showed epigenetic changes in 106 (35%) out of 300 patients. Patients who had no evidence of molecular alterations in the VHL gene were significantly younger than patients who carried some molecular change. Molecular alterations in the VHL gene were not restricted to clear-cell RCCs (ccRCCs). CONCLUSIONS: This is the first report that identifies molecular aberrations in the VHL gene from a South Asian population. The frequency of somatic mutation is lower and that of promoter hypermethylation is higher when compared with data from other parts of the world. The data has important implications in the population-specific application of tailored preventive and therapeutic regimens in non-familial RCCs.

Primary Non-Hodgkin's Lymphoma of Penis Masquerading as a Non-Healing Ulcer in the Penile Shaft.

Primary malignant lymphoma of the male external genitalia is extremely rare and it is even rarer in the penis. Because of its rarity, the possibility of delay in diagnosis and mismanagement is always there. It can present as a nodule, non-healing ulcer, stricture urethra or periurethral abscess. We report a case presenting first a nodule and later on as a non-healing ulcer which was diagnosed by corporal biopsy and managed successfully with chemotherapy.

Whole-pelvis or bladder-only chemoradiation for lymph node-negative invasive bladder cancer: single-institution experience.

PURPOSE: Whole-pelvis (WP) concurrent chemoradiation (CCRT) is the standard bladder preserving option for patients with invasive bladder cancer. The standard practice is to treat elective pelvic lymph nodes, so our aim was to evaluate whether bladder-only (BO) CCRT leads to results similar to those obtained by standard WP-CCRT. METHODS AND MATERIALS: Patient eligibility included histopathologically proven muscle-invasive bladder cancer, lymph nodes negative (T2-T4, N-) by radiology, and maximal transurethral resection of bladder tumor with normal hematologic, renal, and liver functions. Between March 2005 and May 2006, 230 patients were accrued. Patients were randomly assigned to WP-CCRT (120 patients) and BO-CCRT (110 patients). Data regarding the toxicity profile, compliance, initial complete response rates at 3 months, and occurrence of locoregional or distant failure were recorded. RESULTS: With a median follow-up time of 5 years (range, 3-6), WP-CCRT was associated with a 5-year disease-free survival of 47.1% compared with 46.9% in patients treated with BO-CCRT (p = 0.5). The bladder preservation rates were 58.9% and 57.1% in WP-CCRT and BO-CCRT, respectively (p = 0.8), and the 5-year overall survival rates were 52.9% for WP-CCRT and 51% for BO-CCRT (p = 0.8). CONCLUSION: BO-CCRT showed similar rates of bladder preservation, disease-free survival, and overall survival rates as those of WP-CCRT. Smaller field sizes including bladder with 2-cm margins can be used as bladder preservation protocol for patients with muscle-invasive lymph node-negative bladder cancer to minimize the side effects of CCRT.

Histopathology of surgically treated renal tumours in young adults: a developing country perspective.

BACKGROUND: There is no data on the histopathological characteristics of renal tumours in young adults in Pakistan. MATERIALS AND METHODS: We retrospectively analysed 133 young adults (age: 16 to ≤40 years) who underwent nephrectomy for suspected renal cancer from 1994 till July 2010. The demographical and pathological parameters were determined from original surgical biopsy reports and case files, and analysed. RESULTS: The mean age of patients was 33.3 ± 6.2 years. Overall, 121 (88.9%) renal tumours were malignant, and 15 (11%) benign. Among malignant tumours, renal cell carcinoma (RCC) was predominant (100/121; 82.6%). The subtypes of RCC included: clear cell RCC, 84 (84%); papillary RCC, 11 (11%), chromophobe RCC, 3 (3%) and sarcomatoid RCC, 2 (2%). Other malignant tumours included: transitional cell carcinoma (9/121; 7.4%), primitive neuroectodermal tumour (5/121; 4.1%), synovial sarcoma (2/121; 1.6%), Non-Hodgkin's lymphoma (2/121; 1.6%), leiomyosarcoma (1/121; 0.8%), malignant fibrous histiocytoma (1/121; 0.8%) and squamous cell carcinoma (1/121; 0.8%). Among benign tumours, there were 11 cases of angiomyolipoma (11/15; 73.3%), three cases of oncocytoma (3/15; 20%) and one case of schwannoma (1/15; 6.6%). Almost two-third (62.7%) patients had stage I/II tumours, 22 (18.1%) stage III and 23 (19%) stage IV disease at the time of surgery. CONCLUSION: A wide variety of renal tumours is documented in young adults with large size of the tumours and late presentation in our population.

Renal tumors in young adults: a single-center experience from a developing country.

PURPOSE: To determine the pattern and outcome of renal tumors in young adults in a large surgical series in Pakistan. MATERIALS AND METHODS: We retrospectively analyzed 133 young adults (age: ≥ 16 to ≤ 40 years) with 136 renal tumors, who underwent surgical treatment for suspected renal cancer from 1994 till 2010. The clinical and pathological parameters were determined and their impact on final outcome was analyzed. RESULTS: The mean age of the patients was 33.3 ± 6.2 years. Of 136, 121 (88.9%) renal tumors were malignant and 15 (11%) were benign. Among malignancies, 76 (62.7%) patients had stage I or II tumors, 22 (18.1%) stage III, and 23 (19%) stage IV at surgery. The overall cancer-specific survival for malignant tumors at 1, 5, and 10 years was 97%, 83%, and 83%, whereas the cancer-free survival (CFS) was 80%, 63%, and 37%, respectively. Patients with age ≤ 35 years had 1 and 5-year CFS of 83% and 71%, respectively, as compared with 76% and 49% for patients > 35 years (P = .02; odds ratio = 2.3; P = .03). Regarding tumor size, 1 and 5-year CFS for tumors ≤ 10 cm was 93% and 75%, while tumors > 10 cm showed CFS of 56% and 41%, respectively (P = .0001; odds ratio = 4.2; P = .0001). For stage I tumors, CFS at 1 and 5 years was 98% and 84%; for stage II, 82% and 63%; and for stage III, 62% and 50%,respectively. One-year survival for stage IV was 48% only (P = .0001). CONCLUSION: A wide heterogeneity of renal tumors is seen in young adults with delayed presentation.

Primitive neuroectodermal tumor/Ewing's sarcoma in adult uro-oncology: A case series from a developing country.

Peripheral primitive neuroectodermal tumor/Ewing's sarcoma (PNET/EWS) is primarily a tumor of soft tissues and bones. Primary localization of PNET/EWS in genitourinary organs is rare. No data on this localization of PNET/EWS are available in literature from Pakistan. We searched our adult uro-oncology records from 1994 till date and identified all cases of adult genitourinary and adrenal PNET/EWS diagnosed on histology and immunohistochemistry. Their case records were reviewed to obtain data on demographics, presentation, pathologic features, management and outcome. Six cases were found; all were young and had aggressive disease at presentation. Four had renal PNET/EWS. One case each of prostate and adrenal PNET/EWS was seen. Surgery and chemotherapy formed the mainstay of management. Three patients (50%) died during treatment, two were lost to follow-up and one case with renal PNET/EWS showed good initial response to chemotherapy but was later on lost to follow-up. In conclusion, PNET/EWS should be considered in the differential diagnosis of genitourinary malignant tumors in young patients. These tumors are aggressive with poor outcome.

Association of a single-nucleotide polymorphism in the promoter region of the VEGF gene with the risk of renal cell carcinoma.

AIMS: Vascular endothelial growth factor (VEGF) protein plays an important role in tumor development and progression. Polymorphisms in the VEGF gene may lead to over- or underexpression of the protein and may be associated with either risk or progression of malignancy. The aim of this case-control study is to identify and quantify the correlation between VEGF polymorphisms and renal cell carcinoma (RCC). RESULTS: Restriction fragment length polymorphism methods were used for the analysis of VEGF polymorphisms at -2578 and +936 positions in the promoter and 3'-untranslated regions, respectively. The VEGF -2578 A-allele was associated with an increased risk of RCC (odds ratio: 1.6; 95% CI: 1.2-2.3) and A-carrier genotypes were strongly correlated (odds ratio: 2.7; 95% CI: 1.5-4.7) with higher risk. Comparison of VEGF +936 C/T polymorphism between patient and control groups revealed no association with renal carcinoma. Both VEGF -2578 C/A and VEGF +936 C/T polymorphisms showed no significant association with the histopathological parameters of RCC. CONCLUSIONS: This study shows that VEGF -2578 A-allele and A-carrier genotypes are associated with an increased risk of RCC. In groups with higher incidence of RCC, a screening test for this polymorphism may be recommended in conjunction with other established markers.